| Written | 2017-11 | Adriana Zamecnikova |
| Kuwait Cancer Control Center, Kuwait annaadria@yahoo.com |
| Abstract | Translocations and inversions involving the TRA/TRD locus at 14q11.2 and the TRB locus at 7q34 are characteristic anomalies in T-cell acute leukemia/lymphoma. In most instances, juxtaposing promoter and enhancer elements of TCR genes in the proximity of a partner gene results in their dysregulation that may be a critical step toward development of T-cell malignancies. |
| Keywords | T-cell lymphoblastic leukemia/lymphoma, t(7;14)(q34-35;q11), T- cell receptor genes. |
| Identity |
| ICD-Topo | C420,C421,C424 |
| ICD-Morpho | 9837/3 T lymphoblastic leukaemia/lymphoma |
| ICD-Morpho | 9705/3 Angioimmunoblastic T-cell lymphoma |
| Atlas_Id | 3284 |
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| Figure 1. Partial karyotype with t(7;14)(q34;q11). | |
| Clinics and Pathology |
| Note | As translocation breakpoints near terminal regions of chromosomes are difficult to ascertain, included are cases with 7q35 breakpoints, probably involving TRB. |
| Disease | T-cell acute lymphoblastic leukemia (T-ALL) and angioimmunoblastic T-cell lymphoma (AITL). | ||||||||||||||||||||||||||||||||
| Etiology | T-cell malignancies: 4 patients with T-cell acute lymphoblastic leukemia (Kaneko et al., 1988; Secker-Walker et al., 1992; Cauwelier et al., 2006) and 3 with angioimmunoblastic T-cell lymphoma (AITL) (Cosimi et al., 1990) (Table 1.). Table 1. Clinical and genetic testing findings in reported patients with t(7;14)(q34-36;q11).
1. Kaneko et al., 1988; 2. Secker-Walker et al., 1992; 3-4. Cauwelier et al., 2006; 5-7. Cosimi et al., 1990. Abbreviations: M, male; F, female; AITL, Angioimmunoblastic T-cell lymphoma; LN, lymph node; T-ALL, T-cell Acute lymphoblastic leukemia. | ||||||||||||||||||||||||||||||||
| Epidemiology | 2 female and 1 male patients aged 5, 18 and 30 years old (Secker-Walker et al., 1992; Cauwelier et al., 2006). In addition 1 male (Kaneko et al., 1988) and 3 female patients (Cosimi et al., 1990) with 7q35 breakpoints were reported. | ||||||||||||||||||||||||||||||||
| Prognosis | Unknown due to the rarity of reported patients. |
| Cytogenetics |
| Cytogenetics Morphological | Because in translocations with breakpoints in near terminal regions of chromosomes precise breakpoints are often difficult to define, cytogenetic appearance of t(7;14)(q34;q11) may resemble t(7;14)(q35;q11) and rearrangements of TRB at 7q34 may be detected only by using FISH with appropriate probes. Sole anomaly in 2 (Kaneko et al., 1988; Secker-Walker et al 1992), complex karyotype in 1 (Cauwelier et al., 2006) and found as +der(14)t(7;14)(q34;q11),+mar1 in 1 T-ALL patient (Cauwelier et al., 2006). Found in association with complex anomalies including dup(7)(q11q35),t(7;7)(p13;q35),t(7;14)(p13;q11) and t(14;14)(q11;q32) in AITL patients (Cosimi et al., 1990). |
| Genes involved and Proteins |
| Gene Name | TRB (T-cell receptor beta locus) |
| Location | 7q34 |
| Note | Various genes are activated in T-cell malignancies as a consequence of chromosome translocations, inversions and cryptic chromosomal rearrangements involving sequences from the TRB locus such as: t(6;7)(q27;q34) TRB/ MYB, inv(7)(p15q34)/t(7;7)(p15;q34) TRB/ HOXA10, t(7;9)(q34;q34) TRB/ NOTCH1, t(7;10)(q34;q24) TRB/ HOXA11, t(7;11)(q34;p15) TRB/ LMO1, t(7;11)(q34;p13) TRB/ LMO2, t(7;14)(q34;q32.1) TRB/ TCL1A. |
| Gene Name | TRA- TRD |
| Location | 14q11.2 |
| Note | TRA/TRD is a target of chromosomal translocations and inversions including: inv(14)(q11q32.1)/t(14;14)(q11;q32.1) TRA-TRD/TCL1A, t(8;14)(q24.1;q11.2) MYC/TRA-TRD, t(10;14)(q24;q11.2) TLX1/TRA-TRD, t(11;14)(p15;q11.2) LMO1/TRA-TRD and t(11;14)(p13;q11.2) LMO2/TRA-TRD. |
| Result of the chromosomal anomaly |
| Oncogenesis | Translocations between T-cell receptor genes and various oncogenes are a genetic hallmarks of T-cell malignancies. Chromosomal aberrations affecting TCR gene loci mainly involve TRA/TRD at 14q11.2 and TRB at 7q34 and affect various known oncogenes that most often code for transcription factors or proteins involved in transcriptional complexes, the deregulated expression of which is putatively implicated in T-cell oncogenesis. |
| To be noted |
| Additional cases are needed to delineate the epidemiology of this rare entity: you are welcome to submit a paper to our new Case Report section. |
| Bibliography |
| Molecular cytogenetic study of 126 unselected T-ALL cases reveals high incidence of TCRbeta locus rearrangements and putative new T-cell oncogenes |
| Cauwelier B, Dastugue N, Cools J, Poppe B, Herens C, De Paepe A, Hagemeijer A, Speleman F |
| Leukemia 2006 Jul;20(7):1238-44 |
| PMID 16673021 |
| Rearrangements on chromosomes 7 and 14 with breakpoints at 7q35 and 14q11 in angioimmunoblastic lymphadenopathy and IBL-like T-cell lymphoma |
| Cosimi MF, Casagranda I, Ghiazza G, Rossi G, Galvani P |
| Pathologica 1990 Jul-Aug;82(1080):391-7 |
| PMID 2284141 |
| Chromosome translocations involving band 7q35 or 7p15 in childhood T-cell leukemia/lymphoma |
| Kaneko Y, Maseki N, Homma C, Sakurai M, Mizutani S, Takeda T, Shikano T, Fujimoto T, Yaoi K, Shimokawa T |
| Blood 1988 Aug;72(2):534-8 |
| PMID 2900030 |
| Karyotype and T-cell receptor expression in T-lineage acute lymphoblastic leukemia |
| Secker-Walker LM, Campana D, Hawkins JM, Sampson RE, Coustan-Smith E |
| Genes Chromosomes Cancer 1992 Jan;4(1):41-5 |
| PMID 1377008 |
| Citation |
| This paper should be referenced as such : |
| Zamecnikova A |
| t(7;14)(q34;q11) TRA/TRB; |
| Atlas Genet Cytogenet Oncol Haematol. in press |
| On line version : http://AtlasGeneticsOncology.org/Anomalies/t0714q34q11ID1839.html |
| Translocations implicated (Data extracted from papers in the Atlas) |
| t(7;14)(q34;q11) TRA/TRB | |
| External links |
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| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Mon Aug 12 17:00:23 CEST 2019 |
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